Technical considerations in liver transplantation for biliary atresia with situs inversus: A single center experience and a literature review

Abstract

Introduction: In liver transplantation (LT) for biliary atresia (BA) with situs inversus (SI), the surgical procedure is technically challenging due to multiple anatomical variations. The surgical procedures and the outcomes in our patients and reviewed previously-reported cases undergoing LT for BA with SI were evaluated. Method: Between November 2005 and October 2018, 235 children underwent LT with an overall 10-year graft survival of 94.7%. Of these, 6 patients (2.6%) received LT for BA with SI. Result: Living donor LT (LDLT) was applied in all the patients. Vascular anomalies included the absence of the inferior vena cava (IVC) (n=5), preduodenal portal vein (PV) (n=4), and aberrant hepatic artery (HA) (n=1). Hepatic vein (HV) outflow obstruction occurred in one patient with absent IVC and necessitated repositioning of the graft immediately after LDLT. PV stenosis occurred in one patient with preduodenal PV five months after LDLT. Overall patient survival was 80% at 3 years. Forty-nine cases of LT for BA with SI were reviewed. The survival rate was 91.8%, and the vascular complications included HV outflow obstruction in 2.0%, PV complications in 10.2%, and HA thrombosis in 4.1% of the cases. All PV complications occurred in patients with preduodenal PV (5/23; 21.7%) undergoing LDLT. Conclusion: The assessment of the details of the surgical procedures and the information of systemic review demonstrated excellent outcomes of LT for BA with SI, and the incidence of complications associated with vessel anomalies except preduodenal PV was unremarkable.